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Background/purpose: The purpose of this study was to review the authors' 25-year experience with redo pull-through procedures for Hirschsprung's disease including surgical technique and long-term outcome., Methods: From 1974 to now, over 325 patients with Hirschsprung's disease have been treated at C.S. Mott Children's Hospital. This includes 30 patients referred after an unsuccessful pull-through at another hospital and 2 patients with an unsuccessful pull-through from C.S. Mott. All redo pull-throughs (n = 19) were performed in these patients, and their clinical courses are reviewed., Results: Twelve patients required reoperation secondary to a mechanical problem with their first pull-through. The other 7 patients had evidence of residual segments of dilated colon leading to functional failure of their initial operation including 5 patients with documented aganglionic bowel present at the second pull-through. Ten of the patients requiring reoperation initially had an endorectal pull-through (ERPT), 5 had a Duhamel procedure, 3 had a Swenson procedure, and 1 had a Rehbein procedure. Choice of revision was an ERPT in 8 patients in whom an adequate rectal cuff could be developed. Additional redo procedures included a Duhamel in 8 patients and a Swenson in 3 patients. Follow-up ranges from 3 months to 23 years (mean, 13.8 years). There were no deaths in the series, and 1 patient required a third pull-through. All patients who are not neurologically impaired and are over age 3 are continent except one (94%). Stools per day range from 1 to 10 (mean, 3.2)., Conclusions: Redo pull-through operations for Hirschsprung's disease appear to be as effective as primary procedures in terms of continence and stooling frequency. Distinct from other series, we found an ERPT to be the procedure of choice if an adequate rectal cuff was present.

Background/purpose: Regression of a cystic adenomatoid malformation (CAM) in a fetus is well described. Little, however, is known about the postnatal course of these infants. This study attempts to correlate the prenatal course of CAMs with postnatal symptoms, radiological manifestations, and need for surgery., Methods: The clinical course of patients with a CAM diagnosed prenatally were retrospectively reviewed. Inclusion in the study required a prenatal ultrasound scan documenting a CAM., Results: Over 10 years, 14 patients with a CAM were diagnosed prenatally. Six (43%) showed a partial in utero regression. Four patients were symptomatic at birth and underwent a resection as newborns. Ten patients were asymptomatic at birth, and eight of these had normal chest x-rays. Elective resection has been performed in 3 of these 10, and two additional children are scheduled to undergo an excision near 1 year of age. The remaining five patients have undergone follow-up nonoperatively for a mean of 36 +/- 15 months. Of the seven asymptomatic patients not undergoing immediate surgery, only one has shown a slight postnatal regression, despite five of these showing regression in utero. None have become symptomatic., Conclusions: The results suggest that regression of a CAM on prenatal ultrasound scan is common, but this process does not continue after birth. A normal chest x-ray does not indicate complete regression of a CAM; a computed tomography (CT) scan is required to evaluate such patients, and will generally demonstrate a CAM. Asymptomatic patients with a CAM may be followed up nonoperatively with no apparent adverse effects. The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric surgeons.

Objective: The authors investigated risk factors for failure after portoenterostomy for biliary atresia using univariate and multivariable methods., Summary Background Data: Kasai's portoenterostomy has gained worldwide acceptance as the initial surgical therapy for infants with biliary atresia. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly., Methods: The authors analyzed risks for failure in 266 patients treated from 1972 to 1996 by the Kaplan-Meier product limit estimate and Cox proportional hazards model. Failure was defined as death or transplant., Results: Age at surgery, surgical decade, and anatomy of atretic bile ducts were identified as independent risk factors. Five-year survival was 49% and median survival was 15 years when bile drainage was achieved. Sixty-five patients had liver transplants. Mean age at transplant was 5.4 years., Conclusions: The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anatomy of the atretic extrahepatic bile ducts. Liver transplant will salvage patients with failed Kasai with 10-year posttransplant survival of 71%.

In this paper a method is developed which can be used to estimate the body burden of organic hydrophobic chemicals in earthworms. In contrast to the equilibrium partitioning theory, two routes of uptake are incorporated: uptake from interstitial water and dietary uptake. Although many uncertainties still remain, calculations show that for earthworms steady state body burdens are mainly determined by uptake from interstitial water. Under most circumstances, the contribution of dietary uptake is small, except for hydrophobic chemicals (log Kow > 5) in soils with a high organic matter (OM) content of ≈ 20 %. Under those conditions, estimates of the steady state body burden calculated with the equilibrium partitioning model, in which only uptake from interstitial water is taken into account, might result in a small underestimation of the real body burden of chemicals in earthworms.

Constrictive pericarditis is a slowly progressive disabling disease. The diagnosis is easily overlooked because of the striking extracardial signs and symptoms such as abdominal discomfort, general fatigue, cachexia, ascites and oedema. We describe 7 patients with these symptoms in whom the diagnosis was missed during 0.5-17 years. The decisive clue for correct diagnosis appeared to be the raised central venous pressure. This proves the importance of an accurate physical examination. Other findings were: ascites (7/7), hepatomegaly (7/7), oedema (6/7), narrow pulse pressure (less than or equal to 35 mmHg) (5/7), ECG abnormalities (7/7) and pericardial calcifications on the chest X-ray (5/7). In addition we found slightly raised liver enzymes and a protein-losing enteropathy leading to low serum protein levels. These abnormalities are all explained by the alterations in haemodynamics and lymph flow. The only curative therapy is surgical decortication of the heart.

In the assessment of myocardial perfusion by ECG-triggered digital radiography, time parameters are calculated from the time density curve (TDC) and related to blood flow. Recently we developed a method which uses mean transit time (Tmn) as time parameter, and which is in accordance with the original principles of indicator dilution theory. In this approach, variability in vascular volume is excluded and Tmn-1, determined at maximal hyperemia, showed an excellent correlation with maximal flow in animal validation studies. For calculation of Tmn, however, a large part of the descending limb of the TDC has to be known for reliable extrapolation, and especially this part of the curve is subject to variability in image quality in man. Therefore we tested reproducibility of Tmn in 30 arteries in 20 patients. Tmn was derived from the TDCs, obtained from paired studies under identical circumstances with an interval of 10 minutes. Satisfactory images could be obtained in all but one patient. Image processing was performed in an identical way in the paired studies. Reproducibility proved to be excellent for all three coronary arteries. The absolute value of the relative differences between the first and second determination was 7 +/- 7% for the LAD, 6 +/- 3% for LCx and 4 +/- 2% for the RCA (mean +/- SD). Correlation coefficients between both measurements were 0.97, 0.95 and 0.95 for the respective arteries. Therefore, it is concluded that, using this approach, Tmn at maximal hyperemia can be determined reproducibly in man and used for maximal myocardial flow assessment.

Over the last decade it has become more and more obvious that besides anatomical information about the severity of coronary artery stenoses, information about coronary and myocardial blood flow is necessary to understand the functional significance of these obstructions and to evaluate the result of an intervention. Several methods have been proposed for this purpose, each of these having their particular limitations. In this study a new method is shortly described which allows the accurate calculation of relative maximal myocardial perfusion by ECG-triggered digital radiography (videodensitometry), using mean transit time (Tmn) as time parameter; this technique is based on the original physiologic principles of indicator dilution theory. This method was validated in 8 instrumented dogs in which an excellent linear relation was present between 1/Tmn and flow (r = 0.96 +/- 0.03). Although this method does not allow assessment of resting flow and therefore coronary flow reserve (CFR), it provides a means for the reliable comparison of maximal myocardial flow in different situations and it is independent of most factors affecting coronary flow reserve. The ratio between maximal flow after and before an intervention is called maximal flow ratio (MFR) and this concept was applied in a pilot study in man to evaluate PTCA results in 10 patients undergoing elective angioplasty. MFR was compared with the result of exercise testing 24 hours before and 10 days after the angioplasty. MFR greater than or equal to 1.5 was always accompanied by reversal of exercise test result from positive to negative. We conclude that the accurate calculation of relative maximal perfusion of the myocardium is possible by videodensitometry and suggest that comparison of maximal flow after and before an intervention can be valuable in man for functional evaluation of the result of the intervention.

A new approach to angioplasty of a coronary artery bifurcation lesion is described. Via a single guiding catheter, two ultra low-profile balloon-on-wire probes were advanced into the AV sulcus branch and a large obtuse marginal branch of the circumflex artery. The balloons were inflated simultaneously. The technique was easy to perform and remarkably effective.